SUDANESE JOURNAL OF PAEDIATRICS 2017; Vol 17, Issue No.

Original Article
Prune belly syndrome: A report of 15 cases
from Sudan
Abdelmoneim E M Kheir (1), Eltigani M A Ali (1), Safaa A Medani (2), Huda S Maaty (3)
(1) Department of Paediatrics and Child Health, Faculty of Medicine, University of Khartoum and Soba
University Hospital, Sudan.
(2) Department of Pediatrics and Child Heath, Faculty of Medicine, Al Neelain University, Khartoum, Sudan.
(3) Department of Paediatrics, Soba university hospital, Khartoum, Sudan.

ABSTRACT
Prune belly syndrome is a rare congenital patients underwent surgical procedures. At the last
malformation of unknown aetiology, composed follow up visit, 2 (13.4%) patients had normal renal
of a triad of deficient abdominal wall muscle, function tests, 8 (53.3%) ended with chronic kidney
cryptorchidism and urinary tract anomalies. The disease, and 5 died with a mortality rate of 33.3%.
majority of patients have associated pulmonary, Prune belly syndrome is a rare entity with wide
skeletal, cardiac, and gastrointestinal defects. variability in severity and clinical manifestations.
This was a prospective, case finding study The mortality in prune belly syndrome remains
that was conducted in the main paediatric high despite medical and surgical interventions.
hospitals in Khartoum state, during the period
December 2015 to September 2016. A total of
15 patients with prune belly syndrome were
Keywords:
collected. Patients’ characteristics were noted Prune Belly Syndrome; Renal Anomaly;
including socio-demographic data, laboratory Undescended Testis; Abdominoplasty; Sudan.
and radiological investigations and any medical
or surgical intervention. There were 12 males
and 3 females with a male to female ratio of 4:1.
INTRODUCTION
Most of the patients (80%) had hydronephrosis Prune belly syndrome (PBS) (Triad syndrome,
and hydroureter. The study revealed that 60% of Eagle Barret syndrome) is a rare congenital
the patients had associated anomalies, there were anomaly characterized by a triad of deficient
4 (26.6%) with cardiac defects, 3 (20%) with abdominal wall muscle, cryptorchidism and urinary
orthopaedic defects one patient with small bowel tract anomalies, affecting 3.8 per 100,000 live male
volvulus and one patient with cleft lip. 6 (40%) births [1,2]. Over 95% of patients are boys and only
patients received medical intervention and 8 (53%) 3-5% are girls [3]. The cause of PBS is unknown,

Correspondence to: How to cite this article:

Abdelmoneim E. M. Kheir Kheir AEM, Ali EMA, Medani SA, Maaty HS.
Associate Professor, Department of Paediatrics and Prune belly syndrome: A report of 15 cases from
Child Health, Faculty of Medicine, University of Sudan. Sudan J Paediatr 2017; 17(1):42–48
Khartoum and Soba University Hospital, P.O. Box
102, Khartoum, Sudan.
Telephone (mobile): +249 9 12313110, Fax +249
183776295
Email: [email protected]

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SUDANESE JOURNAL OF PAEDIATRICS 2017; Vol 17, Issue No. 1

several aspects of the syndrome, including familial anomalies were also looked for. The morphology of
case reports and a higher incidence in males, the urinary tract was assessed with ultrasonography
suggest that it is influenced by a sex-linked genetic and micturating cysto urethrography (MCUG).
factor, although some have suggested an autosomal Diethylene Triamine Pentaacetic Acid (DTPA)
recessive mode of inheritance [4]. Malformation renal scan was done for selected patients. Repeat
of the urinary system includes different degrees investigations were individualized based on each
of kidney dysplasia, hydronephrosis, dilated and case presentation and progress. Regular outpatient
tortuous ureters, enlarged bladder, sometimes follow up was maintained for all cases during
diverticulum near the vesicoureteric junction, and the nine months period of the study. Glomerular
urethral obstruction [5]. Up to 75% of patients filtration rate (GFR) for children aged 1-18 years
with PBS have associated pulmonary, skeletal, was calculated using the bedside Schwartz equation
cardiac, and gastrointestinal defects [6]. There are 2009 [8]:
few reports from developing countries regarding Estimated glomerular filtration rate (eGFR) =
the pattern of renal involvement and management 0.413 × (height/Scr)
outcome of patients with PBS, but these reports Height is expressed in centimeters.
are deficient due to lack of sufficient follow up to
determine the course of the disease [7]. eGFR = mL/min/1.73 m2
We report on 15 patients with PBS with wide Standardized serum creatinine (Scr) = mg/dL
variability in severity and clinical manifestations, Stage of chronic kidney disease (CKD) was
most of the patients had associated anomalies and determined according to GFR as per the national
the majority had hydronephrosis and hydroureter. kidney foundation guidelines [9]. For infants, less
Despite medical and surgical interventions, the than one year of age we used reference values for
mortality remains high among this group of serum creatinine in different subgroups, where
patients. 2.5th, 10th, 90th and 97.5th percentiles were
the percentiles predicted from the model [10].
METHODS Blood pressure was measured using standard
sphygmomanometer and cuff as well as a Dinamap
This was a prospective, case finding study that monitor. Data was analyzed using Statistical
was conducted in the main paediatric hospitals in Package for Social Sciences (SPSS) for windows
Khartoum state, the capital of Sudan. The study version 16. Frequency analysis for background
was conducted during the period December 2015 variables was conducted.
to September 2016. All children with a clinical and
radiological diagnosis of prune belly syndrome or
one of its variants, males and females, below 18
RESULTS
years of age, were included in the study. A total of 15 The study group comprised 12 males and 3 females
patients with PBS were collected. The diagnosis of with a male to female ratio of 4:1. Regarding the age
PBS was usually evident at birth clinically. Patients distribution, there were 3 patients less than one month of
with prune belly–like variant were also included in age, 7 patients between 1-5 years and 3 above 5 years of
the study, these usually have abdominal wall defect age (Table 1).
without urological involvement. Patients were seen
Seven patients (46.7%) were diagnosed antenatally and
either as inpatients or in the outpatient clinics and
the rest weren’t. There were 14 patients with typical
examined by a senior Registrar or a Consultant PBS and only one patient with prune belly like variant
Paediatrician. who had the typical absent abdominal wall muscle and
A detailed questionnaire was filled out by the normal renal ultrasound. Most of the patients (80%) had
researchers, containing socio-demographic data, hydronephrosis and hydroureter, 13.3% had megacystis
laboratory and radiological investigations and and one patient had a normal renal ultrasound (Table 2).
any medical or surgical intervention done for MCUG was done for 12 out of 15 patients, 66.6% of
these patients. Dysmorphism and any associated the patients had vesicoureteric reflux (VUR), one

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patient had patent urachus plus VUR and 13.3% percentile and one was below the 2.5th percentile.
had posterior urethral valve (PUV) in addition to At last follow up for the same patients, three were
VUR (Table 3). above 97.5th percentile, one on the 90th percentile
Regarding the degree of VUR, one patient had grade and one was between the 10th and 90th percentile.
2, 2 patients (13.3%) had grade 3, 3 patients (20%) GFR at follow up visits for children above one
had grade 4 and 4 patients (26.7%) had grade 5. year of age showed that there was one patient with
normal GFR, one patient with end stage kidney
When the associated anomalies were assessed,
disease (stage 5) and 8 patients with CKD. Table 5
our series revealed that 60% of the patients had
illustrates the GFR and the corresponding stage of
associated anomalies. There were 4 (26.6%) with
CKD in patients above one year of age.
cardiac defects, 3 (20%) with orthopaedic defects
mainly talipes deformity and scoliosis, one patient With regards to the treatment given to these patients,
with small bowel volvulus and one patient with 6 (40%) patients received medical intervention like
cleft lip (Table 4). prophylactic antibiotics, renal replacement therapy
and mechanical ventilation. 8 (53%) patients
DTPA scan was done for 4 patients out of 15 due
underwent surgical procedures, about 50% of the
to availability and affordability, 3 patients had
patients had abdominoplasty and orchidopexy,
severe renal damage on the left kidney and one
one patient had vesicostomy and another one had
patient had severe damage on the right kidney with
cystoscopy with PUV ablation. Table 6 illustrates
poor drainage and mild to normal kidney function
the surgical procedures done.
on the contralateral side. Regarding base line and
follow up serum creatinine for patients less than At the last follow up visit, 2 (13.4%) patients had
one year of age, three of them had serum creatinine normal RFT, 8 (53.3%) ended with CKD, and 5 died
above the 97.5th percentile, one patient on the 90th with a mortality rate of 33.3% among our series.

Table 1- Age distribution of patients with prune belly syndrome

Age Frequency Percentage
0-1 month 3 20.0
>1month - <1year 2 13.3
>1 - <5 years 7 46.7
> 5 year 3 20.0
Total 15 100.0

Table 2- Ultrasound findings in patients with prune belly syndrome

Ultrasound findings Number Percentage
Normal 1 6.7
megacystis only 2 13.3
Hydronephrosis + hydroureter 8 53.3
Hydronephrosis + hydroureter + dysplastic kidney 1 6.7
Hydronephrosis + hydroureter + megacystis 1 6.7
Hydronephrosis + hydrouriter + megacysitic + dysplastic kidney 2 13.3
Total 15 100

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Table 3- Micturating cystourethrogram findings in patients with prune belly syndrome

MCUG findings Number Percentage
Not done 3 13.3
Normal 1 6.7
Megacystis only 1 6.7
Patent urachus + VUR 1 6.7
Megacystis + VUR 2 13.3
PUV + VUR 2 13.3
VUR 5 66.6
Total 15 100
MCUG - Micturating cystourethrogram, PUV - Posterior urethral valve, VUR - Vesicoureteric reflux

Table 4- Associated anomalies in patients with prune belly syndrome

Anomalies Number Percentage
Cardiac defect 4 26.6
ASD 3 –
PDA 1 –
Orthopaedic 3 20
Talipes equiovarus 1 –
Talipes calcaneoovalgum 1 –
Scoliosis 1 –
Gastrointestinal system 1 6.7
Small bowel volvulus 1 –
Others (cleft lip) 1 6.7
No associated anomalies 6 40
Total 15 100
ASD - Atrial septal defect, PDA - Patent ductus arteriosus

Table 5- Glomerular filtration rate at follow visit for patients more than one year of age
Age at follow up GFR (ml/min per 1.73m2) Stage Comment
1 year 30 S III B CKD
16 months 47 S III A CKD
2 years 65 S II CKD
2.25 years 28 S IV CKD
2.50 years 68 S II CKD
2.50 years 118 SI Normal
3 years 41 S III B CKD
6 years 84.7 S II CKD
7 years 24 S IV CKD
10 years 6.7 SV ESKD
Total (n = 10 patients) – – 66.7%
CKD ‑ Chronic Kidney disease, ESKD ‑ End stage kidney disease, GFR ‑ Glomerular filtration rate

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Table 6- Surgical intervention for patients with prune belly syndrome

Intervention Number Percentage
Cystoabdominoplasty and orchidopexy 1 6.7
Abdominoplasty and orchidopexy 2 13.4
Orchidopexy 1 6.7
Vescicostomy 1 6.7
Gastrojejunostomy 1 6.7
Cystoscopy with PUV ablation 1 6.7
Others 1 6.7
No intervention 7 46.6
Total 15 100

DISCUSSION
Prune Belly syndrome (PBS) was first described by bowel volvulus, this is lower than what is reported
Frolich in 1839 and it is a complex malformation in the literature of 20-30% which is thought to result
disorder with wide variability in severity and clinical from an insufficient fixation of the mesentery to the
manifestations [11]. Though rare, cases of familial back of the abdominal wall [16, 17]. Twenty percent
PBS have been reported [4]. None of our patients of the patients in our series had orthopaedic defects
however has any positive family history. mainly talipes deformity and scoliosis, which is lower
Our series revealed that male to female ratio in than 45-63% reported associated orthopaedic defects
children with PBS was 4:1 which indicates a higher [18], More severe deformities of the musculoskeletal
female preponderance than what is reported in the system such as limb deformities and arthrogryposis
literature, this is hard to explain but as this is a case appear to be secondary to oligohydramnios causing
finding study, some cases might have been missed. compression of the developing fetus [19].
The PBS is 20 times more common in males than The current case series showed that most of the
female and this has been attributed to the more patients (80%) had hydronephrosis and hydroureter,
complex morphogenesis in males than in females which is similar to other reports, as hydronephrosis
(Figure 1) [12, 13]. is a common finding and is usually detected during
The present study showed that only 46.7% of patients antenatal care visits [20]. About 50% of the patients
with PBS were diagnosed antenatally, which might in our series had abdominoplasty and orchidopexy.
indicate either poor antenatal care attendance or lack In addition to the cosmetic effect of abdominoplasty,
of accuracy of antenatal ultrasonography. Prenatal there is an additional advantage of improvement in
diagnosis plays a key role in early detection of PBS, bladder emptying with a marked reduction in post
with prenatal ultrasonography; PBS can usually be void residual urine and improved bowel function
diagnosed in the second trimester of pregnancy [14]. (Figure 2) [21]. It is recommended that orchidopexy
Our series revealed that 60% of the patients had should be performed as early as possible since
associated anomalies, which is compatible with the endocrine function is preserved and there is a
what is reported in the literature [6]. 26.6% of malignant potential [22].
patients with PBS had cardiac defects mainly atrial From our series, the overall mortality rate of 33.3%
septal defects; this is higher than 10% reported compares with the worldwide mortality rate of 20-
cardiovascular involvement [15]. Given this 30%, mortality in PBS is usually due to pulmonary
association, it is recommended that all prune belly hypoplasia and renal failure [7]. The prognosis in PBS
patients should undergo cardiovascular evaluation. is variable and depends on the degree of pulmonary
Our series showed that 6.7% of the patients had hypoplasia, associated renal anomalies, and degree of
associated gastrointestinal anomalies mainly small renal compromise [23].

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Figure 1- Female baby with Prune belly Figure 2- A child with Prune belly syndrome
syndrome who had abdominoplasty

CONCLUSION ACKNOWLEDGEMENTS
PBS is a rare entity worldwide with wide variability in The authors would like to thank the families of the
severity and clinical manifestations, most of the patients had children mentioned in this report for consent of
associated anomalies and the majority had hydronephrosis participation, publication and using the images.
and hydroureter. The mortality in PBS remains high despite Thanks are also extended to the administration of the
medical and surgical interventions. Prenatal diagnosis plays Paediatric hospitals in Khartoum state, Sudan.
a key role in early detection of PBS.

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